tailieunhanh - Báo cáo y học: "Unique presentations and chronic complications in adult cystic fibrosis: do they teach us anything about CFTR?"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học 'Respiratory Research cung cấp cho các bạn kiến thức về ngành y đề tài:Unique presentations and chronic complications in adult cystic fibrosis: do they teach us anything about CFTR. | http content 1 3 133 Commentary Unique presentations and chronic complications in adult cystic fibrosis do they teach us anything about CFTR Michael P Boyle Johns Hopkins University School of Medicine Baltimore Maryland USA o 3 I Received 16 October 2000 Accepted 24 October 2000 Published 16 November 2000 Respir Res 2000 1 133-135 Current Science Ltd Print ISSN 1465-9921 Online ISSN 1465-993X Abstract The increase in numbers of adults with cystic fibrosis CF has allowed us to identify previously unrecognized chronic complications of CF as well as appreciate unique presentations of cystic fibrosis-related diseases. Do these chronic complications and unique presentations provide us with new insight into cystic fibrosis transmembrane conductance regulator CFTR function Current data suggest that the chronic complications reveal mainly the effect of a long-term absence of previously recognized CFTR functions. In contrast the unique presentations provide new insight into the role of CFTR in different tissues. Keywords congenital bilateral absence of the vas deferens cystic fibrosis cystic fibrosis transmembrane conductance regulator malignancy pancreatitis Introduction One of the most striking trends in cystic fibrosis CF over the past few decades has been the marked increase in expected life span with median survival improving from less than 10 years in the 1960s to more than 30 years now. Currently more than one-third of all individuals with CF are over the age of 18 1 and trends suggest that in the next decade adults will account for nearly half of the CF population. It is accepted that new insights into the basic pathophysiology of CF might allow continued increases in survival but is the converse also true Will increased survival allow greater insight into the basic pathophysiology of CF This question can be asked because with increased length of survival has come the recognition of previously underappreciated CF-related complications .